AB0732 Abatacept as therapy option in systemic sclerosis (SSc) patients after years of Grazer- protocol treatment- Our Experience
Authors: M. D’orazio, M. Daher, A. Lackner, S. Kielhauser, S. Zenz, J. Gretler, J. Thiel, H. P. Brezinsek, F. Fürst-Moazedi
Journal: Annals of the Rheumatic Diseases (2022)

Background Previous studies have provided evidence that T cells may play a significant role in the pathogenesis of systemic sclerosis (SSc) and observational studies reported that Abatacept, which interferes with T cells activation, appeared to be safe and effective in SSc-patients with skin and muscle involvement. So far, there was no significant effective treatment for SSC-ILD. We have recently demonstrated the effectiveness of intensified –RTX- long term treatment (“Grazerprotocol” with 500 mg RTX in week 0 and 2, every 3 months +/- MMF) in SSc patients with severe organ involvement and/or progressive form non responding to methotrexate or cyclophosphamide. However, no data are available concerning how long Patients with a stable disease need to be treated. B-cell-depletion therapy over years for SSC in remission might represent a risk factor for infections. Additionally, the parenteral administration is associated with an increase in health care and patients are bound to regular hospital visits, which is a limiting factor for life quality. Objectives We aimed to investigate if subcutaneous Abatacept could be used as a maintenance treatment in SSc-patients reaching a stable disease activity after a treatment with RTX administered according to our Grazer protocol over several years. Methods In this retrospective analysis, we retrieved data from 20 patients who fulfilled the diagnosis criteria for systemic sclerosis (SSc) according to the European League Against Rheumatism Scleroderma Trial and Research standards, who started a treatment with abatacept (ABA) after years of 500 mg RTX therapy every three months +/- MMF (Grazer protocol). The following clinical parameter were evaluated: modified Rodnan Skin Score (mRSS), Systemic Sclerosis Activity Score (SScAS), Systemic Sclerosis Severity Score (SScSS) and lung diffusing capacity for carbon monoxide (DLCO). Lab parameters like IgG, ANA, ENA and inflammation parameters were routinely assessed. Clinical data from baseline visit (BSL) (before ABA treatment start) and follow-up visit (FU) (after 6 months of treatment) were collected. Results We included 20 SSc patients in this retrospective analysis who changed from RTX to Abatacept. The majority were female (n=16; 94.1%), with a mean age ±SD of 54.8 years ±11 and an average disease duration of 7.7 years ±4.5. In 17.9% (n=4) treatment needed to be stopped due to disease flare after three months (lung n=2, skin and tendons n=2). However, interestingly, Abatacept further decreased significantly mRSS between baseline visit and follow-up visit regarding the affected skin of the fingers. Thus, the mRSS went from 3.9±3.4 to 2.5±1.8 (p&lt;0.05). As expected, no significant difference in the EUSTAR-SScAS, or ScSS or DLCO was found. No infections were observed during the abatacept follow-up period and Immunoglobulins remained within the normal range. Conclusion Abatacept might be a feasible option as a maintenance therapy after intensive immunomodulation with RTX and may give our patients the possibility to further improve their quality of life. The fact that 4 patients experienced a relapse after switching to Abatacept warrants further studies to find prognostic factors. Disclosure of Interests None declared